KERATOPROSTHESIS KERATOPROSTHESIS IN HIGHIN HIGH--RISK PEDIATRIC RISK PEDIATRIC

CORNEAL TRANSPLANTATIONCORNEAL TRANSPLANTATIONEsen Karamursel Akpek, MD

The Wilmer Eye Institute, Baltimore, MarylandJames V. Aquavella,

University of Rochester Eye Institute, Rochester, New York

IntroductionIntroduction

•• Prognosis poorer for pediatric PKPrognosis poorer for pediatric PK•• FactorsFactors

Surgical technique challengingSurgical technique challengingHeightened immune responseHeightened immune response

IntroductionIntroduction

•• Congenital Corneal Congenital Corneal OpacificationOpacification–– Anterior Segment Anterior Segment DysgenesesDysgeneses–– Congenital GlaucomaCongenital Glaucoma–– SclerocorneaSclerocornea–– Birth TraumaBirth Trauma

IntroductionIntroduction

•• Other highOther high--risk factors:risk factors:–– Uncontrolled glaucoma Uncontrolled glaucoma –– Combined surgery (CE, Combined surgery (CE, ppvppv, tube shunt) , tube shunt) –– Age <1 yearAge <1 year–– Repeat graftRepeat graft(Yang et al. Ophthalmology 1999) (Yang et al. Ophthalmology 1999) (Comer et al. JAAPOS 2001)(Comer et al. JAAPOS 2001)

IntroductionIntroduction

•• AmblyopiaAmblyopia: limits visual acuity: limits visual acuity–– Irregular astigmatismIrregular astigmatism–– AphakiaAphakia–– Difficulty in visual rehabilitationDifficulty in visual rehabilitation

••60% clear graft / VA only 30% achieved 60% clear graft / VA only 30% achieved 20/400 or better20/400 or better

((StultingStulting RD, et al. 1984 and Dana MR, et al 1005)RD, et al. 1984 and Dana MR, et al 1005)

IntroductionIntroduction

•• KeratoprosthesisKeratoprosthesis–– Favorable outcome in multiple graft Favorable outcome in multiple graft

failure casesfailure cases((YaghoutiYaghouti et al. Cornea 2001)et al. Cornea 2001)

–– Low incidence of Low incidence of endopthalmitisendopthalmitis in nonin non--cicatricialcicatricial corneal opacitiescorneal opacities((NouriNouri et al. Am J et al. Am J OphthalmolOphthalmol 2001)2001)

Advantages of KAdvantages of K--propro

•• KeratoprosthesisKeratoprosthesis does not does not opacify/vascularizeopacify/vascularize

•• Spherical anterior shapeSpherical anterior shape•• Can correct for refractive errors including Can correct for refractive errors including

aphakiaaphakia

PurposePurpose

•• To propose keratoprosthesis as an To propose keratoprosthesis as an alternative procedure to PK in highalternative procedure to PK in high--risk pediatric casesrisk pediatric cases

PatientsPatients

•• N= 15 (Baltimore=5 and Rochester=10)N= 15 (Baltimore=5 and Rochester=10)•• Age: mean 36 Age: mean 36 mosmos (2 (2 mosmos to 11 years)to 11 years)•• M/F: 8/7M/F: 8/7•• DxDx: Congenital corneal opacities: Congenital corneal opacities

Surgical TechniqueSurgical Technique

Surgical TechniqueSurgical Technique

•• DohlmanDohlman--Doane type I Doane type I keratoprosthesiskeratoprosthesis

Surgical TechniqueSurgical Technique

•• Donor corneal button oversized 1.0 mmDonor corneal button oversized 1.0 mm•• KeratoprosthesisKeratoprosthesis + + BaerveldtBaerveldt tube shunt tube shunt

+ pars + pars planaplana vitrectomyvitrectomy + + lensectomylensectomy

ResultsResults•• FollowFollow--up: 1up: 1-- 28 28 mosmos (median 10 (median 10 mosmos))•• Boston KBoston K--pro (n=13) and pro (n=13) and AlphaCorAlphaCor (n=2 both (n=2 both

failed)failed)•• Retinal detachment and phthisis of the globe Retinal detachment and phthisis of the globe

(n=1, Boston K(n=1, Boston K--pro)pro)•• One patient had traumatic dehiscence of the One patient had traumatic dehiscence of the

Boston KBoston K--pro and underwent second onepro and underwent second one•• Rest are doing wellRest are doing well

Case OneCase One

•• 20 20 mosmos female with Petersfemale with Peters’’ syndromesyndrome•• S/P multiple glaucoma procedures, and S/P multiple glaucoma procedures, and

PKsPKs, OU, OU•• Phthisis OD with NLPPhthisis OD with NLP•• Failed PK (x2), OS with LP Failed PK (x2), OS with LP

•• Initial EUAInitial EUA

––Opaque graft with Opaque graft with epiepi defect; defect; microcorneamicrocornea, IOP over 40, IOP over 40

––BB--Scan; aphakia with dense Scan; aphakia with dense epiretinal membrane epiretinal membrane

––KK--pro + PPV + tube shunt pro + PPV + tube shunt placement, OSplacement, OS

––No intraNo intra--op complicationsop complications

•• 9 weeks post9 weeks post--op, op, choroidalschoroidals with RD with RD •• Membrane peeling, SBP, fluidMembrane peeling, SBP, fluid--air air

exchange, silicone oil tamponade, exchange, silicone oil tamponade, replacement Kreplacement K--propro

•• Retina remained attached 28Retina remained attached 28 months months following repairfollowing repair

Case 5Case 5

•• 5 year old F with Peters anomaly5 year old F with Peters anomaly•• S/P multiple S/P multiple PKsPKs, OU, OU•• VA OD: NLP OS: LP VA OD: NLP OS: LP •• GlaucomaGlaucoma•• OD OD phthisicalphthisical

Case 5Case 5

•• Underwent Boston KUnderwent Boston K--pro and tube shuntpro and tube shunt•• No complicationsNo complications•• Developed Developed retroprostheticretroprosthetic membrane 10 membrane 10

mosmos after surgery requiring YAG after surgery requiring YAG •• Did well with a Did well with a f/uf/u 17 17 mosmos

DiscussionDiscussion

•• KeratoprosthesisKeratoprosthesis in highin high--risk pediatric risk pediatric cases may maintain clear visual axis cases may maintain clear visual axis during the formative early years of during the formative early years of visual developmentvisual development

DiscussionDiscussion

ConsConsGlaucoma management altered Glaucoma management altered following keratoprosthesis; monitor following keratoprosthesis; monitor optic nervesoptic nervesSurgery and postSurgery and post--operative care operative care challengingchallengingRequires team of experienced Requires team of experienced surgeonssurgeons

ConclusionConclusion

•• Keratoprosthesis may be a viable Keratoprosthesis may be a viable alternative to corneal transplantation alternative to corneal transplantation in pediatric highin pediatric high--risk casesrisk cases

•• Future studies with longer followFuture studies with longer follow--up up are needed prior to recommending are needed prior to recommending keratoprosthesis in highkeratoprosthesis in high--risk pediatric risk pediatric casescases